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NS_BTRAN > Anterior Horn, PERIPHERAL NERVES, NMJ Disorders > Flashcards

Anterior Horn, PERIPHERAL NERVES, NMJ Disorders Flashcards

1
Q

ALS*, SMA, polio, West Nile are what class of nerve disorder?

A

Anterior horn cell disorders

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2
Q

Cervical & lumbar spine; brachial & lumbosacral plexus are what class of nerve disorder?

A

Radiculopathies and plexopathies

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3
Q

Mononeuropathies* – carpal tunnel, ulnar & peroneal palsy.

Polyneuropathies – genetic (CMT), diabetes, systemic illness, vitamin deficiency, toxic.

Are what type of disorders?

A

Neuropathies

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4
Q

Myasthenia*, LEMS, botulism, organophosphate poisoning are what class of disorder?

A

Neuromuscular junction disorders

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5
Q

What are the Myopathies?

A

Dystrophies*, myositis, metabolic, toxic & endocrine myopathies

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6
Q

UMN vs LMN?

__1__– spastic tone; incr. reflexes; emotional
__2__– decr. tone & reflexes; atrophy

A
  1. UMN
  2. LMN
BOTH: Weakness
Rapidity, location, progression
Atrophy, hypertrophy, deformities
Other
Sensory loss; cramps; pain
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7
Q

What is the name of a autoimmune disorder, ab to NMJ acetylcholine receptor?

A

Myasthenia Gravis

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8
Q

What is the Tx for Myasthenia Gravis?

A
  1. Cholinesterase inhibitor → temporary increase in strength and improve decrement following repetitive nerve stimulation
    Edrophonium (IV)
    Pyridostimine (oral)
  2. Immunosuppression:
    Prednisone
    Immunosuppressive agents (azathioprine, mycophenolate mofitil)
  3. Plasma exchange, IVIG infusion
  4. Thymectomy
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9
Q

How do you Dx Myasthenia Gravis?

A

Diagnosis via presence of ACHR antibodies in serum

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10
Q

What are the clinical features of Myasthenia Gravis?

A

Diagnosis via presence of ACHR antibodies in serum

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11
Q

What is the disease that is one of the hereditary motor and sensory neuropathies, a group of varied inherited disorders of the peripheral nervous system characterised by progressive loss of muscle tissue and touch sensation across various parts of the body.

A

Charcot–Marie–Tooth disease

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12
Q

What is Amyotrophic Lateral Sclerosis (ALS)?

A

Pathologic changes:
Progressive weakness and wasting due to degeneration of brainstem and spinal cord lower motor neurons
Coexisting spasticity and hyperreflexia due to degeneration of upper motor neurons
Hyperreflexia + pathological reflexes (Hoffman, crossed adductor, Babinski)

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13
Q

What are the clinical features of Amyotrophic Lateral Sclerosis (ALS)?

A

WEAKNESS

Typically sporadic
Foot drop
Speech may be slurred or spastic
Cognitive defects may be seen
Normal sensory exam
Diaphragm weakness
Impaired swallowing → aspiration pneumonia
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14
Q

ALS frequency _____ (increase or decrease) with age?

A

increases.

ALS affects male more, reasons unknown.

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15
Q

How do you Tx ALS?

A 
Disease-specific: pathogenesis based
riluzole: of proven benefit
many past and pending trials of agents
complementary/alternative medicine (CAM)
widely used
symptom-specific
improve QOL and longevity
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16
Q

How does Becker Muscular Dystrophy different from DMD?

A

Differs from Duchenne, only in that it has a later onset, has more benign course, and survival is longer.

Mental impairment seen less often.

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17
Q

What is the genetic defect in DMD?

A

Genetic defect: XR, deletion, duplication, and point mutations on X chromosome.

Only boys.

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18
Q

What are the clinical features of DMD?

A
  • Clumsy waddling gait from first walking.
  • Accentuation of lumbar lordosis → protuberant abdomen.
  • Calf enlargement.
  • Right heel cords, toe walking.
  • Gower’s maneuver - trouble rising from floor.
  • Subnormal intelligence.
  • Eye movements, swallowing and sensation unaffected.
  • Increasing proximal weakness with age
  • Eventually wheelchair bound, and succumb to respiratory or heart failure by late teens or 20s.
19
Q

What is the only drug that helps with ALS? Does it treat ALS?

A 
riluzole: of proven benefit
many past and pending trials of agents
complementary/alternative medicine (CAM)
widely used symptom-specific.
improve QOL and longevity.

Does not cure!

20
Q

What is the best drug you can use for muscle cramps? (no longer available in the US)

A

quinine

21
Q

dysphagia: ?

A

Difficulty swallowing

22
Q

dysarthria: ?

A

difficult or unclear articulation of speech that is otherwise linguistically normal.

23
Q

What are the (2) subtypes of Charcot–Marie–Tooth disease?

A

CMT1A

CMT2

24
Q

Charcot-Marie-Tooth Disease that presents as slow nerve conduction velocities + demyelinating neuropathy: Hereditary motor and sensory neuropathy.

Is of which subtype?

A

CMT1A

25
Q

Charcot-Marie-Tooth Disease that presents as normal nerve conduction velocities + axonal degeneration.
Hereditary motor and sensory neuropathy.

Is of which subtype?

A

CMT2

26
Q

What is the genetic defect of Charcot-Marie-Tooth Disease? (2)

A
  1. AD (CMT1 and CMT2)
    CMT1A = Duplication in DNA containing peripheral myelin protein gene (PMP22)
  2. AR (CMT4)
27
Q

What are the clinical features of Charcot-Marie-Tooth Disease?

A

1) Onset of walking normal, but distal (hands and feet) weakness and sensory loss develop slowly over first two decades of life, not usually confined to wheelchair.
2) Impaired/delayed walking as infants, confined to wheelchair later in life.
3) Adult onset, does not appear until age 40

28
Q

PMP22 gene is ~ with what disorder?

A

CMT disease

29
Q

Describe the CMT1A/HNPP mutation?

A

A 1.4 Mb duplication on chr 17 (PMP22 gene) causes CMT1A.

  - Distal weakness, sensory loss and deformities
  - 10% are de novo mutations.
  • Repeats on either side of gene (CMT1A REPS) are hot spot for recombination
  • Deletion of the same locus causes HNPP; Focal weakness and sensory loss.
30
Q

What are the classifications of diabetic neuropathies?

A
  • Sensory or sensorimotor distal polyneuropathy
    Stocking/glove numbness and burning pain
    Foot drop and weak hand muscles
  • Autonomic neuropathy
    Hypotension, diarrhea, impotence, urinary retention
- Mononeuropathy
Cranial nerve (3, 6,7) or peripheral (median, ulnar, peroneal
  • Lumbosacral plexopathy
    Pelvic girdle pain; asymmetric hip flexor weakness
31
Q

what is the most common subtype of diabetic neuropathies?

A

Sensory or sensorimotor distal polyneuropathy.

 - Stocking/glove numbness and burning pain.
 - Foot drop and weak hand muscles
32
Q

In Carpal Tunnel, what park of the hand is atrophy?

A

The Thenar (hypothenar)

33
Q

What is the Tx of diabetic neuropathy?

A

Good metabolic control of diabetes.

Foot hygiene to avoid ulceration & infection
Night light; well fitting shoes.

Pain management
anticonvulsants, antidepressants, analgesics.

Autonomic symptoms
Diarrhea- codeine, diphenoxylate
Hypotension –fluorocortisone, midodrine
Urinary retention –regular voiding; suprapubic pressure.

Make sure to TAKE CARE OF FOOT!

34
Q

What is the initial complaint of diabetic neuropathy?

A

Initial complaints: numbness, burning in feet → spreads up legs, into hands.

  • Weakness of foot dorsiflexor muscles → slapping foot drop gait.
  • Grip strength and fine hand dexterity diminished
35
Q

What are the positive exam findings of diabetic neuropathy??

A
  • Pin sensation loss in “stocking-glove” distribution.
  • Loss of position, vibration, and light touch.
  • Loss of pain and temperature sensation.
  • Decreased reflexes.
36
Q

What is the autonomic dysfunction ~ with diabetic neuropathy?

A

Postural hypotension, diarrhea, impotence, urinary retention, increased sweating

37
Q

Explain the effects of cholinesterase inhibitors on nondepolarizing vs. depolarizing neuromuscular blocking agents.

A

AChEIs can help overcome a block of AChRs by curare (nondepolarizing blocking agent), but AChEIs potentiate effects of succinylcholine (depolarizing neuromuscular blocking agent).

38
Q

What is a tensilon? And what does a positive tensilon test = ?

A

Tensilon inhibits the breakdown of acetylcholine so that it accumulates and has a prolonged effect.

Effects include miosis; increased intestinal and skeletal muscle tone; bronchial constriction; bradycardia; increased salivation, lacrimation, and sweating.

Myasthenia gravis.

39
Q

In Myasthenia gravis, what happens to the thymus?

A

abnormal hyperplasia of the thymus. (vs. normal involution with age)

40
Q

involution: ?

A

the shrinkage of an organ in old age or when inactive, e.g., of the uterus after childbirth.

41
Q

What is Succinylcholine and its MOA?

A

Succinylcholine: N-M receptor agonist (2-ACh molecules joined)
Initial stimulation → muscle fasciculations
Depolarization does not allow repolarization and subsequent AP → flaccid paralysis
Flaccid paralysis is POTENTIATED by AChEIs

42
Q

Explain what happens with brief versus prolonged stimulation of nicotinic cholinergic receptors.

A
  • Prolonged receptor activation prevents the muscle cell membrane from repolarizing, thus preventing subsequent AP → flaccid paralysis.
  • Brief stimulation allows depolarization, repolarization, and then finally a subsequent AP to take place.
43
Q

Plexopathy: ?

A

Plexopathy is a disorder affecting a network of nerves, blood vessels, or lymph vessels.[1] The region of nerves it affects are at the brachial or lumbosacral plexus. Symptoms include pain, loss of motor control, and sensory deficits

44
Q

Mononeuropathy: ?

A

Mononeuropathy is a type of neuropathy that only affects a single nerve.[5] Diagnostically, it is important to distinguish it from polyneuropathy because when a single nerve is affected, it is more likely to be due to localized trauma or infection.

NS_BTRAN (21 decks)

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